Background: Portal biliopathy producing intrahepatic and extrahepatic biliary ductal abnormalities from portal hypertension, particularly with extrahepatic portal vein obstruction (EHPVO) is common. A majority of these patients are asymptomatic, but occasionally there is symptomatic biliary obstruction, and cholangitis and choledocholithiasis.
Objective: To explore the principles of diagnosis and treatment of portal biliopathy.
Data sources: To review the literature of portal biliopathy.
Conclusions: Endoscopic sphincterotomy, stone extraction and supportive drainage could effectively relieve cholangitis when jaundice is associated with common bile duct stones. Definitive decompressive portal-system vein shunting operation and choledocho-jejunostomy are sometimes required when biliary obstruction is recurrent and progressive.